title: Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA).
abstract: BACKGROUND
  For almost 30 years, anticoagulation has been recommended for patients with idiopathic pulmonary arterial hypertension (IPAH). Supporting evidence, however, is limited, and it is unclear whether this recommendation is still justified in the modern management era and whether it should be extended to patients with other forms of pulmonary arterial hypertension (PAH).
  
  
  RESULTS
  We analyzed data from Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA), an ongoing European pulmonary hypertension registry. Survival rates of patients with IPAH and other forms of PAH were compared by the use of anticoagulation. The sample consisted of 1283 consecutively enrolled patients with newly diagnosed PAH. Anticoagulation was used in 66% of 800 patients with IPAH and in 43% of 483 patients with other forms of PAH. In patients with IPAH, there was a significantly better 3-year survival (P=0.006) in patients on anticoagulation compared with patients who never received anticoagulation, albeit the patients in the anticoagulation group had more severe disease at baseline. The survival difference at 3 years remained statistically significant (P=0.017) in a matched-pair analysis of n=336 IPAH patients. The beneficial effect of anticoagulation on survival of IPAH patients was confirmed by Cox multivariable regression analysis (hazard ratio, 0.79; 95% confidence interval, 0.66-0.94). In contrast, the use of anticoagulants was not associated with a survival benefit in patients with other forms of PAH.
  
  
  CONCLUSIONS
  The present data suggest that the use of anticoagulation is associated with a survival benefit in patients with IPAH, supporting current treatment recommendations. The evidence remains inconclusive for other forms of PAH.
  
  
  BACKGROUND
  http://www.clinicaltrials.gov. Unique identifier: NCT01347216.
authors.affiliation: Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover, Germany (K.M.O., M.M.H.); Department of Pneumology, University Hospitals of Leuven, Leuven, Belgium (M.D.); University of Giessen and Marburg Lung Center (UGMLC), German Center for Lung Research (DZL), Hannover, Germany (H.A.G., H.T.); Department of Rheumatology and Clinical Immunology, Charité University Hospital, and Epidemiology unit, German Rheumatism Research Centre, Berlin, Germany (D.H.); Clinic of Internal Medicine, University Hospital, Zurich, Switzerland (R.S.); University Hospital Heidelberg, Heidelberg, Germany (E.G.); Medical Clinic I, Clinic Loewenstein, Loewenstein, Germany (G.S.); Clinic III for Internal Medicine (Cardiology), and Center for Molecular Medicine Cologne (CMMC), University of Cologne, Germany (S.R.); Internal Medicine I, University Hospital Carl Gustav Carus of Technical University Dresden, Dresden, Germany (M. Halank); Department of Internal Medicine, Respiratory Medicine and Cardiology, Mission Medical Hospital, Würzburg, Germany (M. Held); Department of Internal Medicine II, Division of Pneumology, University Medical Center Regensburg, Regensburg, Germany (T.J.L.); Department of Internal Medicine V, University of Munich, Munich, Germany (J.B.); University Medical Center Hamburg-Eppendorf, Center of Oncology, Department of Respiratory Medicine, Hamburg, Germany (H. Klose); LungenClinic Grosshansdorf, Germany (M.C.); Clinic of Internal Medicine, Department of Respiratory Medicine, Ernst Moritz Arndt University of Greifswald, Greifswald, Germany (R.E.); Department of Cardiology, DRK Kliniken Berlin Köpenick, Berlin, Germany (C.F.O.); Department of Cardiovascular and Respiratory Diseases, Sapienza, University of Rome; Rome, Italy (C.D.V.); Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy (L.S.); Department of Pulmonary Diseases, VU University Medical Center, Amsterdam, The Netherlands (A.V.-N.); Dep
authors.forename: Karen M
authors.initials: KM
authors.lastname: Olsson
authors.forename: Marion
authors.initials: M
authors.lastname: Delcroix
authors.forename: H Ardeschir
authors.initials: HA
authors.lastname: Ghofrani
authors.forename: Henning
authors.initials: H
authors.lastname: Tiede
authors.forename: Doerte
authors.initials: D
authors.lastname: Huscher
authors.forename: Rudolf
authors.initials: R
authors.lastname: Speich
authors.forename: Ekkehard
authors.initials: E
authors.lastname: Grünig
authors.forename: Gerd
authors.initials: G
authors.lastname: Staehler
authors.forename: Stephan
authors.initials: S
authors.lastname: Rosenkranz
authors.forename: Michael
authors.initials: M
authors.lastname: Halank
authors.forename: Matthias
authors.initials: M
authors.lastname: Held
authors.forename: Tobias J
authors.initials: TJ
authors.lastname: Lange
authors.forename: Juergen
authors.initials: J
authors.lastname: Behr
authors.forename: Hans
authors.initials: H
authors.lastname: Klose
authors.forename: Martin
authors.initials: M
authors.lastname: Claussen
authors.forename: Ralf
authors.initials: R
authors.lastname: Ewert
authors.forename: Christian F
authors.initials: CF
authors.lastname: Opitz
authors.forename: C Dario
authors.initials: CD
authors.lastname: Vizza
authors.forename: Laura
authors.initials: L
authors.lastname: Scelsi
authors.forename: Anton
authors.initials: A
authors.lastname: Vonk-Noordegraaf
authors.forename: Harald
authors.initials: H
authors.lastname: Kaemmerer
authors.forename: J Simon R
authors.initials: JS
authors.lastname: Gibbs
authors.forename: Gerry
authors.initials: G
authors.lastname: Coghlan
authors.forename: Joanna
authors.initials: J
authors.lastname: Pepke-Zaba
authors.forename: Uwe
authors.initials: U
authors.lastname: Schulz
authors.forename: Matthias
authors.initials: M
authors.lastname: Gorenflo
authors.forename: David
authors.initials: D
authors.lastname: Pittrow
authors.forename: Marius M
authors.initials: MM
authors.lastname: Hoeper
mesh_terms: D000368:Aged; D000925:Anticoagulants / Q000008:administration & dosage* / Q000009:adverse effects; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005500:Follow-Up Studies; D006470:Hemorrhage / Q000139:chemically induced / Q000401:mortality; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000401:mortality*; D015994:Incidence; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011379:Prognosis; D011446:Prospective Studies; D012042:Registries; D013927:Thrombosis / Q000401:mortality* / Q000517:prevention & control*
chemical_list: D000925:Anticoagulants
keywords: anticoagulation; pulmonary hypertension; survival; therapy